Question

Hypoplastic uterus with streak gonads is a feature of

• A. Testicular feminization syndrome
• B. Triple X syndrome
• C. Adrenogenital syndrome
• D. Turner syndrome

Hint:

Think about which condition has ovarian failure but no testes to block uterus formation.

Answer 1

The Correct Option is D

This question tests the classic picture of streak gonads plus a small uterus, which points straight to one well known chromosomal disorder.

1. Testicular feminization syndrome: Here the person is genetically male (46,XY) with working testes hidden in the abdomen or groin. These testes make anti-Mullerian hormone, so the uterus never forms at all. Since a uterus is absent, not small, this does not fit.
2. Triple X syndrome: Women with an extra X chromosome (47,XXX) almost always have normal ovaries, a normal uterus, and normal periods, so gonadal failure is not a typical feature.
3. Adrenogenital syndrome: This is congenital adrenal hyperplasia in a 46,XX person. The ovaries and uterus are normal internally; only the external genitalia look different because of excess androgens.
4. Turner syndrome: A single X chromosome (45,X) means the ovaries cannot survive fetal life and shrink into streak gonads. Because no testes ever form, the uterus does develop from the Mullerian ducts, but stays small and childlike due to a lack of estrogen support.

The pairing of streak gonads with a hypoplastic, not absent, uterus is unique to Turner syndrome.

Let's summarize:

• Streak gonads plus a present but small uterus signals ovarian failure with normal Mullerian development, as in Turner syndrome.
• An absent uterus points to a testes driven cause like testicular feminization, not Turner syndrome.

So the correct answer is Turner syndrome.